Patients with Darier's Disease, hyperkeratosis follicularis, have been said to have involvement of the lid margins and an isolated corneal opacity. We have examined 18 patients and report that 14 have peripheral, deep epithelial, grouped lesions characteristic of this disease. Fourteen of the patients also have central dendritiform irregularity of the corneal surface. Other ocular changes involve the corneal periphery with pannus and marginal ulcers or hyperkeratotic areas of the lid margins.